Gastrointestinal manifestations in myotonic muscular dystrophy

来源 :World Journal of Gastroenterology | 被引量 : 0次 | 上传用户:delicioussmoke
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Myotonic dystrophy(MD)is characterized by myotonicphenomena and progressive muscular weakness.Involvement of the gastrointestinal tract is frequentand may occur at any level.The clinical manifestationshave previously been attributed to motility disorderscaused by smooth muscle damage,but histologicevidence of alterations has been scarce and conflicting.A neural factor has also been hypothesized.In the upperdigestive tract,dysphagia,heartburn,regurgitation anddyspepsia are the most common complaints,while inthe lower tract,abdominal pain,bloating and changesin bowel habits are often reported.Digestive symptomsmay be the first sign of dystrophic disease and mayprecede the musculo-skeletal features.The impairmentof gastrointestinal function may be sometimes sogradual that the patients adapt to it with little awarenessof symptoms.In such cases routine endoscopic andultrasonographic evaluations are not sufficient andtargeted techniques(electrogastrography,manometry,electromyography,functional ultrasonography,scintigraphy,etc.)are needed.There is a low correlationbetween the degree of skeletal muscle involvement andthe presence and severity of gastrointestinal disturbanceswhereas a positive correlation with the duration of theskeletal muscle disease has been reported.The drugs recommended for treating thegastrointestinal complaints such as prokinetic,anti-dyspeptic drugs and laxatives,are mainly aimed atcorrecting the motility disorders.Gastrointestinal involvement in MD remains acomplex and intriguing condition since many importantproblems are still unsolved.Further studies concentratingon genetic aspects,early diagnostic techniques and thedevelopment of new therapeutic strategies are neededto improve our management of the gastrointestinalmanifestations of MD. Myotonic dystrophy (MD) is characterized by myotonicphenomena and progressive muscular weakness. Involvement of the gastrointestinal tract is frequent and may occur at any level. The clinical manifestations have previously been attributed to motility disorderscaused by smooth muscle damage, but histologiceidence of alterations has been scarce and conflicting . A neural factor has also been hypothesized. The upper digestive tract, dysphagia, heartburn, regurgitation and dyspepsia are the most common complaints, while inthe lower tract, abdominal pain, bloating and changesin bowel habits are often reported. Digestive symptomsmay be the first sign of dystrophic disease and mayprecede the musculo-skeletal features. The impairment of gastrointestinal function may be sometimes with small awareness of symptoms in patients such as with small awareness of symptoms. In such cases routine endoscopic andultrasonographic evaluations are not sufficient andtargeted techniques (electrogastrography, manometry, electromyography, function al ultrasonography, scintigraphy, etc.) are needed. Here are a low correlation between the degree of skeletal muscle involvement and the presence and severity of gastrointestinal disturbances where as a positive correlation with the duration of the shockletal muscle disease has been reported.The drugs recommended for treating thegastrointestinal complaints such as prokinetic, anti-dyspeptic drugs and laxatives, are mainly aimed atcorrecting the motility disorders. Gastrointestinal involvement in MD remains acomplex and intriguing condition since many important adaptations are still unsolved. Future Research concentrating on genetic aspects, early diagnostic techniques and the development of new therapeutic strategies are neededto improve our management of the gastrointestinalmanifestations of MD.
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